Transcript
HAEMORRHAGIC DISORDERS IN
NEWBORNPRESENTED BY :
VRUTI PATEL,
HAEMORRHAGIC DISEASES
OF THE NEWBORN
It is a syndrome characterized by spontaneous internal or external bleeding. In neonates, there is decreased activity of several clotting factors and diminished platelet function.
CAUSES
• Abnormalities of clotting factors
• Deficiencies of vitamin k dependent factors – II, VII, IX, X. it usually occurs between D2 and D5 and common in preterm and breast fed babies. Deficiency of protein C is also responsible
• Drugs – received by mother during pregnancy – phenytoin, coumarin compounds, salicylates (affects vit K function).
• DIC – due to infection, Anoxia, shock.
2. Disturbances of clotting- Related to DIC due to infection, shock, anoxia,
NEC, renal vein thrombosis, use of IV canula.
3. Inherited abnormalities of C.F.
a. X-LINKED RECESSIVE DISEASES-
i. Hemophilia-A : Factor VIII deficiency.
ii. Hemophilia-B : Factor IX deficiency.
b. AUTOSOMAL DOMINANT DISEASES:
i. Von Willebrand disease – Deficiency of VWF which is a carrier of factor VIII & as a platelet aggregation agent.
c. AUTOSOMAL RECESSIVE DISEASES:
I. Severe factor VII & factor XIII deficiency – intracranial hemorrhage in neonates
II.Factor XI deficiency – unpredictable bleeding during surgery/trauma.
III. VWD Type III
B. Platelet problems:
1. Qualitative disorders:- Glanzman’s thrombasthenia.- Bernard-Soulier syndrome- Platelet type VWD
2. Quantitive disorders:
- Immune thrombocytipenia
- Matrnal Preeclampsia, HELLP syndrome or severe uteroplacental insuffuciency.
- DIC due to infection or asphyxia.
- Inherited bone marrow failure syndromes : Fanconi anemia & congenital amegakaryocytic thrombocytopenia
- Congenital leukemia
- Inherited thrombocytopenia syndromes : gray platelet syndrome
- Macrothrombocytopenias : May-Hegglin syndr.
- Platelet consumption in clots/ vascular disorders eg. Vascular malformations, NEC.
C. Vascular origin:
- Pulmonary haemorrhage
- A-V malformations
- CNS haemorrhage
- Hemangiomas.
DIAGNOSTIC WORKUP
A.History- Family h/o bleeding disorders
- Maternal medications
- Pregnancy & birth history
- Maternal h/o infant with bleeding disorder
- Any medications, procedures, anomalies in infant
B. Examination:First diagnose whether the infant is Sick or Well
1.Sick infant:
- DIC
- Bacterial/ viral infections.
2. Well infant:- Vit K deficiency- Isolated C.F. deficiencies- Immune thrombocytopenia- Maternal blood in infant’s GIT.
3. Patchiae, ecchymosis, mucosal bleeding: Platelet problem
4. Large bruises: DIC, C.F deficiencies, liver diseases
5. Enlarged spleen : Possible congenital infections or erythroblastosis.
6. Jaundice : Sepsis, liver diseases, resorption of large hematoma.
C. Laboratory tests: 1.Apt test :
- To rule out maternal blood in infant’s GIT
- Done in otherwise well infant with only GI bleeding.
2. PBS :
- DIC- fragmented RBCs
- Congenital macrothrombocytopenias – large platelets.
3. PT
4. APTT
5. D-Dimer assays: Measure fibrin degradation products in DIC & Liver diseases causing defective clearing of fibrin split products.
6. Specific factor assays & Von Willebrand assay: For patients with + ve family h/o.
TREATMENT OF BLEEDING
A. Inj Vitamin K1 (Aquaminophyton)
- 1 mg IV or IM if not given at birth.
- Infants on TPN
- Infants on Antibiotics > 2 weeks: at least 0.5mg Vit K weekly.
- Preferred rather than FFP for prolonged PT & PTT, FFP should be reserved for emergencies.
B. FFP:- 10ml/kg IV for active bleeding - Repeated 8-12 hrly as needed.- Replaces C.F. immediately.
C. Platelets:- 1 Unit of platelet raises count by 50,000-
10,000/mm3.- Platelet count slowly decreases if stores 3-5
days.
D. Fresh whole blood:
- 10ml/kg
- Can be repeated after 6-8 hrs as needed.
E. Clotting factor concetrates
- Severe VWD :
- VWF containing plasma derived factor VIII concetrate.
- Known deficiency of factor VIII or IX : Recombinent DNA derived factor VIII and IX concetrate
F. Disorders due to problems other than hemostatic proteins :
- Rule out the underlying possibilities- eg. Infection, Liver rupture, catheter, NEC.
G. T/t of specific disorders :
1.DIC :- Treat the underlying cause i.e. sepsis, NEC- Make sure that Vit K1 has been given.
- Platelets/ FFP to keep platelet counts > 50,000/ml and to stop bleeding.
- If bleeding persists,
i. Exchange transfusion with fresh whole blood /Packed RBC/Platelets/FFP
ii. Continuous transfusion with platelets, packed RBCs or FFP as needed.
iii. For hypofibrinogenemia : Cryoprecipitate (10ml/kg)
top related